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cardiomyopathy pediatric

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https://www.readbyqxmd.com/read/28070248/childhood-cardiomyopathies-a-study-in-tertiary-care-hospital-in-upper-egypt
#1
Mohamed Abd Elaal Bakeet, Montaser Mohamed Mohamed, Ahmed Ahmed Allam, Rania Gamal
INTRODUCTION: Cardiomyopathy (CMP) is defined by the World Health Organization (WHO) as a disease of the myocardium associated with cardiac dysfunction. An understanding of CMP is very important, as it is a common cause of heart failure in children, and the most common indication for heart transplantation in children older than one year, but data on CMP in Egypt are scarce. The aim of this study was to determine the number, risk factors, clinical presentation, complications and outcome of different types of childhood cardiomyopathies in Sohag University Hospital...
November 2016: Electronic Physician
https://www.readbyqxmd.com/read/28040191/left-ventricular-function-in-children-and-adolescents-with-arrhythmogenic-right-ventricular-cardiomyopathy
#2
Paweena Chungsomprasong, Robert Hamilton, Wietske Luining, Meena Fatah, Shi-Joon Yoo, Lars Grosse-Wortmann
The aim of this study was to determine if left ventricular (LV) contractility is reduced in children with arrhythmogenic right ventricular cardiomyopathy (ARVC). For this retrospective study, children and adolescents undergoing a workup for ARVC were characterized according to the revised Task Force Criteria (rTFC). LV strain, rotation, and torsion were measured by feature-tracking cardiovascular magnetic resonance imaging (CMR). Of 142 pediatric patients, 41% had no, 23% possible, 20% borderline, and 16% definite ARVC...
December 2, 2016: American Journal of Cardiology
https://www.readbyqxmd.com/read/28039505/factors-supporting-cardiomyopathy-screening-among-at-risk-adult-survivors-of-pediatric-malignancies
#3
Cheryl L Cox, Liang Zhu, Rohit P Ojha, Brenda D Steen, Susan Ogg, Leslie L Robison, Melissa M Hudson
PURPOSE: Anthracyclines and chest irradiation place adult survivors of childhood cancer at risk of cardiomyopathy; many survivors do not obtain the recommended screening. Based on our recent clinical trial, the addition of telephone counseling to a printed survivorship care plan more than doubled survivors' risk-based screening. Here, we sought to measure the impact of specific factors targeted in the intervention for their impact on survivors' screening participation. METHODS: Study population-survivors participating in a randomized longitudinal intervention trial...
December 30, 2016: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
https://www.readbyqxmd.com/read/28033083/the-hemodynamic-effects-and-safety-of-repetitive-levosimendan-infusions-on-children-with-dilated-cardiomyopathy
#4
Pertti Suominen, Niklas Mattila, Olle Nyblom, Paula Rautiainen, Maila Turanlahti, Otto Rahkonen
BACKGROUND: Limited treatment options are available for children with decompensated dilated cardiomyopathy (DCM), while they wait for either functional recovery or heart transplantation. We evaluated the safety of repetitive levosimendan infusions and short-term and long-term impacts of the therapy in this patient population. METHODS: Eighty-one repetitive levosimendan infusions administered to 20 patients with DCM at severe or end stage of the disease in the pediatric intensive care unit were analyzed retrospectively...
January 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28029746/respiratory-manifestations-in-38-patients-with-alstr%C3%A3-m-syndrome
#5
Caroline Boerwinkle, Jan D Marshall, Joy Bryant, William A Gahl, Kenneth N Olivier, Meral Gunay-Aygun
OBJECTIVES: Alström syndrome (AS) is a rare, multi-system condition characterized by retinal degeneration, sensorineural hearing loss, obesity, insulin-resistant diabetes, hypertriglyceridemia, cardiomyopathy, hepatorenal disease, and recurrent respiratory infections. It belongs to a group of genetic disorders known as primary ciliopathies, which includes autosomal dominant and recessive polycystic kidney diseases, as well as Joubert and Bardet-Biedl syndromes. Prior studies have suggested phenotypic overlap between primary ciliopathies affecting the non-motile, sensory cilia, and primary ciliary dyskinesia (PCD), a motile ciliopathy characterized by respiratory tract disease...
December 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27999550/the-use-of-berlin-heart-excor-vad-in-children-less-than-10-kg-a-single-center-experience
#6
Arianna Di Molfetta, Fabrizio Gandolfo, Sergio Filippelli, Gianluigi Perri, Luca Di Chiara, Roberta Iacobelli, Rachele Adorisio, Isabella Favia, Alessandra Rizza, Giuseppina Testa, Matteo Di Nardo, Antonio Amodeo
Objective: Despite the improvement in ventricular assist device (VAD) therapy in adults and in adolescents, in infant population only Berlin Heart EXCOR (BHE) is licensed as long term VAD to bridge children to Heart Transplantation (HTx). Particularly demanding in terms of morbidity and mortality are smallest patients namely the ones implanted in the first year of life or with a lower body surface area. This work aims at retrospective reviewing a single center experience in using BHE in children with a body weight under 10 kg...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27986330/making-the-quick-diagnosis-a-case-of-neonatal-shock
#7
Mike Gardiner, Timothy K Ruttan, Andrew J Kienstra, Matthew Wilkinson
BACKGROUND: The work-up and initial management of a critically ill neonate is challenging and anxiety provoking for the Emergency Physician. While sepsis and critical congenital heart disease represent a large proportion of neonates presenting to the Emergency Department (ED) in shock, there are several additional etiologies to consider. Underlying metabolic, endocrinologic, gastrointestinal, neurologic, and traumatic disorders must be considered in a critically ill infant. Several potential etiologies will present with nonspecific and overlapping signs and symptoms, and the diagnosis often is not evident at the time of ED assessment...
December 13, 2016: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27966803/yield-of-screening-echocardiograms-during-pediatric-follow-up-in-survivors-treated-with-anthracyclines-and-cardiotoxic-radiation
#8
Michael B Spewak, Rebecca S Williamson, Ann C Mertens, William L Border, Lillian R Meacham, Karen J Wasilewski-Masker
BACKGROUND: Guidelines published by the Children's Oncology Group recommend screening echocardiograms for childhood cancer survivors exposed to anthracyclines and/or cardiotoxic radiation. This study aims to assess risk factors for cardiac late effects while evaluating the overall yield of screening echocardiograms. PROCEDURE: Demographics, exposures, and echocardiogram results were abstracted from the medical records of survivors diagnosed at ≤ 21 years old and ≥ 2 years off therapy who were exposed to anthracyclines and/or potentially cardiotoxic radiotherapy...
December 14, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27933640/tricuspid-annular-plane-systolic-excursion-as-a-marker-of-right-ventricular-dysfunction-in-pediatric-patients-with-dilated-cardiomyopathy
#9
Ericka S McLaughlin, Curtis Travers, William L Border, Shriprasad Deshpande, Ritu Sachdeva
BACKGROUND: Right ventricular systolic dysfunction (RVSD) is a predictor of outcomes in dilated cardiomyopathy (DCM) in adults, but little is known in children. Tricuspid annular plane systolic excursion (TAPSE) has emerged as a reliable tool to assess RVSD. We sought to determine the prevalence and prognostic significance of RVSD using TAPSE in children with DCM. METHODS: The first echocardiogram at the time of diagnosis with DCM was analyzed to obtain left ventricular ejection fraction (LVEF) and 2D TAPSE...
December 9, 2016: Echocardiography
https://www.readbyqxmd.com/read/27933370/doxorubicin-induced-chronic-dilated-cardiomyopathy-the-apoptosis-hypothesis-revisited
#10
REVIEW
Cynthia Kankeu, Kylie Clarke, Egle Passante, Heinrich J Huber
The chemotherapeutic agent doxorubicin (DOX) has significantly increased survival rates of pediatric and adult cancer patients. However, 10% of pediatric cancer survivors will 10-20 years later develop severe dilated cardiomyopathy (DCM), whereby the exact molecular mechanisms of disease progression after this long latency time remain puzzling. We here revisit the hypothesis that elevated apoptosis signaling or its increased likelihood after DOX exposure can lead to an impairment of cardiac function and cause a cardiac dilation...
December 8, 2016: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
https://www.readbyqxmd.com/read/27931578/pretransplant-left-ventricular-dysfunction-adversely-affects-perioperative-outcomes-in-pediatric-liver-transplantation-a%C3%A2-retrospective-observational-study
#11
D-M Jang, I-G Jun, Y-J Moon, W-J Shin, J-G Song, G-S Hwang
BACKGROUND: Although left ventricular diastolic dysfunction (LVDD) is a pronounced feature of adult cirrhotic cardiomyopathy and a major predictor of poor outcomes following liver transplantation (LT), little is known about if pretransplant cardiac dysfunction affects perioperative outcomes in pediatric LT. METHODS: We retrospectively evaluated pretransplant clinical and Doppler echocardiographic data for 45 consecutive pediatric LT recipients who were treated between 2007 and 2013 (median age = 15 months; interquartile range = 9 to 78 months)...
December 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27931092/the-influence-of-race-and-common-genetic-variations-on-outcomes-after-pediatric-heart-transplantation
#12
D J Green, M M Brooks, G J Burckart, R E Chinnock, C Canter, L J Addonizio, D Bernstein, J K Kirklin, D C Naftel, D M Girnita, A Zeevi, S A Webber
Significant racial disparity remains in the incidence of unfavorable outcomes following heart transplantation. We sought to determine which pediatric post-transplantation outcomes differ by race and whether these can be explained by recipient demographic, clinical, and genetic attributes. Data were collected for 80 black and 450 non-black pediatric recipients transplanted at 1 of 6 centers between 1993 and 2008. Genotyping was performed for 20 candidate genes. Average follow-up was 6.25 years. Unadjusted five-year rates for death (p=0...
December 8, 2016: American Journal of Transplantation
https://www.readbyqxmd.com/read/27925367/transient-severe-tricuspid-regurgitation-after-transplantation-of-an-extremely-oversized-donor-heart-in-a-child-does-size-matter-a-case-report
#13
J Birnbaum, S M Ulrich, R Schramm, C Hagl, A Lehner, M Fischer, N A Haas, B Heineking
In pediatric heart transplantation, the size of the donor organ is an important criterion for organ allocation. Oversized donor hearts are often accepted with good results, but some complications in relation to a high donor-recipient ratio have been described. Our patient was transplanted for progressive heart failure in dilated cardiomyopathy. The donor-to-recipient weight ratio was 3 (donor weight 65 kg, recipient weight 22 kg). The intra-operative echocardiography before chest closure showed excellent cardiac function, no tricuspid valve regurgitation, and a normal central venous pressure...
December 7, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27910218/octreotide-use-and-safety-in-infants-with-hyperinsulinism
#14
Ann W McMahon, Gerold T Wharton, Paul Thornton, Diva D De Leon
BACKGROUND: Octreotide is a synthetic peptide analog of naturally occurring somatostatin. Octreotide is used off-label in children <6 years of age for hyperinsulinism, chylothorax, and gastrointestinal bleeding. There is a lack of controlled data on efficacy or potential adverse events from this off-label use. METHODS: Three pediatric hospitals participated in this study. Patients were hospitalized January 2007-December 2010 and administered octreotide for congenital hyperinsulinism (CHI) at least 1 day...
December 2, 2016: Pharmacoepidemiology and Drug Safety
https://www.readbyqxmd.com/read/27890770/fibrosis-and-fibrotic-gene-expression-in-pediatric-and-adult-patients-with-idiopathic-dilated-cardiomyopathy
#15
Kathleen C Woulfe, Austine K Siomos, Hieu Nguyen, Megan SooHoo, Csaba Galambo, Brian L Stauffer, Carmen Sucharov, Shelley Miyamoto
BACKGROUND: While fibrosis seems to be prognostic for adverse outcomes in adults with idiopathic dilated cardiomyopathy (IDC), little is known about the prevalence and development of fibrosis in pediatric IDC hearts. We hypothesize there is less activation of fibrosis at a molecular level in pediatric IDC hearts than in the failing adult heart. METHODS AND RESULTS: Pediatric hearts were analyzed histologically to determine the prevalence of fibrosis. Left ventricular tissue from adult and pediatric IDC hearts and adult and pediatric non-failing (NF) hearts were subjected to qRT-PCR to study the expression of important mRNAs that affect fibrosis...
November 24, 2016: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/27884629/urgent-listing-exceptions-and-outcomes-in-pediatric-heart-transplantation-comparison-to-standard-criteria-patients
#16
Ryan R Davies, Michael A McCulloch, Shylah Haldeman, Samuel S Gidding, Christian Pizarro
BACKGROUND: United Network for Organ Sharing (UNOS) policy enables listing exceptions to avoid penalizing patients with waitlist mortality not captured by standard criteria. Outcomes among patients listed by exception have not been analyzed. METHODS: We performed a retrospective analysis of pediatric (≤17 years of age, n = 4,706) listings (2006 to 2015) for primary, isolated heart transplantation within the UNOS data set, assessing Status 1A exception (n = 211, 4...
October 1, 2016: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/27882422/predictors-of-pharmacological-therapy-of-ectopic-atrial-tachycardia-in-children
#17
Haiyan Ge, Xiaomei Li, Haiju Liu, He Jiang
Ectopic atrial tachycardia (EAT) is a relatively common type of supraventricular tachycardia in the pediatric population, and it can be resistant to antiarrhythmic drugs and lead to tachycardia-induced cardiomyopathy (TIC) if not properly managed. The purpose of this study was to determine the predictors of the response to pharmacological therapy in children with EAT. From January 2009 to April 2014, 115 children were admitted to our hospital with a diagnosis of EAT and placed on antiarrhythmic drugs. We examined the clinical history, response to therapy, and follow-up of the children...
November 24, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27878632/utility-of-echocardiography-in-the-assessment-of-left-ventricular-diastolic-function-and-restrictive-physiology-in-children-and-young-adults-with-restrictive-cardiomyopathy-a-comparative-echocardiography-catheterization-study
#18
Thomas D Ryan, Peace C Madueme, John L Jefferies, Erik C Michelfelder, Jeffrey A Towbin, Jessica G Woo, Rashmi D Sahay, Eileen C King, Roberta Brown, Ryan A Moore, Michelle A Grenier, Bryan H Goldstein
The aim of the study is to determine the utility of echocardiography in the assessment of diastolic function in children and young adults with restrictive cardiomyopathy (RCM). RCM is a rare disease with high mortality requiring frequent surveillance. Accurate, noninvasive echocardiographic measures of diastolic function may reduce the need for invasive catheterization. Single-center, prospective, observational study of pediatric and young adult RCM patients undergoing assessment of diastolic parameters by simultaneous transthoracic echocardiogram (TTE) and invasive catheterization...
November 23, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27867675/a-case-of-thyroid-storm-associated-with-cardiomyopathy-and-poststreptococcal-glomerulonephritis
#19
Lisa J Underland, Gerson A Vallencia Villeda, Abhijeet Pal, Leslie Lam
Thyroid storm has a high mortality rate and is often associated with a precipitating factor such as intercurrent illness or infection. It is rare in pediatric patients. Cardiac disease in hyperthyroidism mostly manifests itself as tachycardia but more serious cardiac findings have also been described. A 5-year-old male with recent strep throat infection presented with dilated cardiomyopathy, hematuria, and symptoms and lab findings consistent with severe hyperthyroidism. He was diagnosed with thyroid storm secondary to concurrent Graves' disease and poststreptococcal glomerulonephritis (PSGN)...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27865440/hemoglobin-of-12-g-dl-and-above-is-not-associated%C3%A2-with-increased-cardiovascular-morbidity%C3%A2-in-children-on-hemodialysis
#20
Michelle N Rheault, Julia T Molony, Thomas Nevins, Charles A Herzog, Blanche M Chavers
In adults on chronic hemodialysis, achieving a hemoglobin concentration of 12g/dl and above with erythropoiesis stimulating agents leads to increased cardiovascular events and mortality, but this may not be true in children. Therefore, we conducted a retrospective cohort study of pediatric patients (under 18) from the Centers for Medicare and Medicaid Services End Stage Renal Disease (ESRD) Clinical Performance Measures (CPM) project (2000 to 2008) merged with the United States Renal Data System. Hemoglobin was determined from the Clinical Performance Measures data, and beginning annually on January 1st of the next year, patients were followed for up to 1 year...
January 2017: Kidney International
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